ILD

Question 1

What are the potential findings of a patient presenting with ILD?

Answer 1

Hands/face: clubbing, cyanosis, RA/SLE (red scaly rash often with knuckles, Raynaud’s, vasculitis lesions)/systemic sclerosis (waxy tight skin, sclerodactyly - prayer sign, calcinosis), cushingoid appearance, grey skin from amiodarone

Chest: fine inspiratory crackles that do not alter with coughing, possibility of single lung transplant (one lung with signs, ipsilateral thoracotomy scar with normal underlying lung)

Question 2

How would you investigate a patient presenting with possible ILD?

Answer 2

HPC, FH, smoking & occupational hx including any allergen expsoures, travel hx

Bedside spirometry
- looking for restrictive pattern which is a proportional reduction in both FVC and FEV1 and a FEV1:FVC ratio >0.8
(+ formal lung function tests)
- reduced TLC
- Reduced TLco and Kco (due to damaged gas exchange surface)

Urine dip: blood/protein indicating multisystem involvement

Bloods
FBC, UE, LFT, CRP, ESR, bone profile, autoimmune panel (ANA, anti-dsDNA, ACE, RF, CCP)
ABG if hypoxic

CXR

High res CT

Bronchoalveolar lavage (mainly to exclude infection before giving immunosuppressants)

Question 3

What are the findings expected on high resolution CT?

Answer 3

Bi-basal sub-pleural honeycombing indicative of usual interstitial pneumonia (hallmark of IPF)

Widespread ground glass shadowing more likely to be non-specific interstitial pneumonia which is more characteristic of autoimmune disease

Apical fibrosis: think sarcoid, ABPA, old TB, hypersensitivity pneumonitis (allergen in history), langerhan cell histiocytosis (expected if has widespread rashes, bony lumps, swollen gums, pituitary disorders)

Question 4

What are the differentials for basal fibrosis?

Answer 4

UIP - IPF

Asbestosis

Connective tissue disease

Aspiration (asymmetric)

Question 5

What are the treatment options for ILD?

Answer 5

Dependent on cause

MDT approach
- patient education
- smoking cessation
- allergen and trigger avoidance
- pulmonary rehab?

Medical
- widespread ground glass infiltrate very responsive to immunosuppression, 80% 5-year survival
- avoid combination of steroids + azathioprine as PANTHER trial indicated increased morbidity with this combination

• UIP - no response to immunosuppression 80% 5-year mortality
• IPF - patient needs referral to NHSE recognised ILD service for consideration of anti-fibrotic agent when FEV1 50-80% predicted
  i.e. pirfenidone, nintedanib

Surgical
Single lung transplant

Question 6

Draw flow volume loops comparing normal, obstructive and restrictive lung disease?

Answer 6

Question 7

What are the differentials for apical fibrosis?

Answer 7

TRASH

TB
Radiation
Ank spond/ ABPA
Sarcoidosis
Histoplasmosis/ Histiocytosis /Hypersensitivity pneumonitis